Clinical features of dermatomyositis associated with anti-MDA5 antibodies by age

Koichi Yamaguchi1, Aya Yamaguchi1, Yuji Onuki1

  • 1Department of Allergy and Respiratory Medicine, Gunma University Graduate School of Medicine, Gunma, Japan.

Modern Rheumatology
|March 10, 2020
PubMed
Abstract

Insights

Age significantly impacts outcomes for anti-melanoma differentiation-associated gene 5 (MDA5) autoantibody-positive dermatomyositis (DM) patients. Older individuals (≥60 years) face worse prognoses, particularly with rapidly progressive interstitial lung disease (ILD).

Area of Science:

  • Rheumatology
  • Pulmonology
  • Immunology

Background:

  • Anti-melanoma differentiation-associated gene 5 (MDA5) autoantibodies are associated with interstitial lung disease (ILD) in dermatomyositis (DM).
  • Age at onset is a known poor prognostic factor in anti-MDA5 positive DM-ILD.
  • The specific impact of age on clinical presentation and outcomes in anti-MDA5 positive DM remains incompletely understood.

Purpose of the Study:

  • To investigate the influence of age on clinical features, treatment, and prognosis in anti-MDA5 autoantibody-positive DM patients.
  • To compare outcomes between younger (<60 years) and older (≥60 years) patient cohorts.
  • To identify specific challenges and mortality factors in elderly anti-MDA5 positive DM patients.

Main Methods:

  • Retrospective analysis of 40 anti-MDA5 autoantibody-positive DM patients.
  • Stratification of patients into two age groups: <60 years and ≥60 years.
  • Comparison of clinical characteristics, laboratory findings, high-resolution computed tomography (HRCT) data, treatment strategies, complications (infections), and survival rates between groups.

Main Results:

  • Older patients (≥60 years) exhibited significantly fewer mucocutaneous symptoms like arthralgia/arthritis and skin ulceration compared to younger patients.
  • The older cohort demonstrated significantly higher mortality rates.
  • Older patients were more frequently complicated by rapidly progressive interstitial lung disease (RP-ILD), necessitating combination immunosuppressive therapy, which proved often ineffective, alongside challenges in infection control.

Conclusions:

  • Age is a critical determinant of clinical phenotype and prognosis in anti-MDA5 autoantibody-positive DM.
  • Elderly patients (≥60 years) with anti-MDA5 positive DM have a poorer prognosis, characterized by a higher risk of RP-ILD and increased mortality.
  • Standard combination immunosuppressive therapies may be less effective for RP-ILD in older DM patients, highlighting the need for age-specific treatment strategies.

Related Concept Videos

Myocarditis II: Clinical Features and Diagnostic Tests01:27

Myocarditis II: Clinical Features and Diagnostic Tests

Myocarditis is an inflammation of the heart muscle. The symptoms vary widely, encompassing asymptomatic presentations to severe, acute manifestations.Clinical PresentationAsymptomatic cases: In some instances, myocarditis may be asymptomatic, with the infection resolving without intervention. These cases often go undetected unless discovered incidentally through diagnostic imaging or tests conducted for other reasons.General Early Symptoms: Early symptoms of myocarditis are non-specific and can...
193
Myasthenia Gravis: Overview and Treatment01:20

Myasthenia Gravis: Overview and Treatment

Myasthenia gravis is a neuromuscular transmission disorder characterized by weakness and increased fatigability of skeletal muscles. It is an autoimmune disease affecting approximately one in 2000 people, where antibodies against the α1 subunit of nicotinic acetylcholine receptors are produced.
These antibodies interfere with the function of the nicotinic receptors in three ways: by binding to the receptor and disrupting acetylcholine binding; by causing cross-linking of receptors which...
2.6K
Myasthenia Gravis: Diagnostic Tests01:15

Myasthenia Gravis: Diagnostic Tests

Myasthenia gravis is an autoimmune condition affecting neuromuscular transmission, causing generalized weakness in skeletal muscles. Initial diagnoses rely on patients' signs, symptoms, and medical history. The challenge lies in distinguishing myasthenia from other muscular dystrophies. An important diagnostic feature is the significant improvement of symptoms after administering anticholinesterase inhibitors.
The edrophonium test is a diagnostic tool for myasthenia gravis. It involves...
1.8K
Rheumatic Heart Disease II: Clinical Manifestations and Diagnostic Studies01:22

Rheumatic Heart Disease II: Clinical Manifestations and Diagnostic Studies

The key clinical manifestations of Rheumatic heart disease (RHD) include several distinct cardiac symptoms.Carditis, a hallmark of acute rheumatic fever, involves inflammation of the heart's endocardium, myocardium, and pericardium. Chronic RHD often results from recurrent episodes of carditis. Its symptoms include the following:Murmurs are caused by valvular damage, especially to the mitral and aortic valves. Mitral stenosis or regurgitation is common, with characteristic heart murmurs...
365
Myocarditis I: Introduction01:21

Myocarditis I: Introduction

Myocarditis is inflammation of the myocardium, which is the muscular layer of the heart.EtiologyMyocarditis has a diverse etiology, including a wide range of infectious and non-infectious causes:Infectious CausesViral: Common viruses include Coxsackie A and B, adenovirus, parvovirus B19, enteroviruses, and influenza A.Bacterial: Examples include infections caused by Streptococcus, Staphylococcus, and Mycoplasma species.Rickettsial: Infections like Rocky Mountain spotted fever can result in...
284
Endocarditis II: Clinical Features of Infective Endocarditis01:25

Endocarditis II: Clinical Features of Infective Endocarditis

Endocarditis can present various clinical features depending on the causative organism and the patient's underlying health conditions. Initially, the clinical features of infective endocarditis develop gradually, presenting with nonspecific symptoms that can be easily mistaken for other illnesses.General SymptomsEarly symptoms of infective endocarditis are fever, chills, weakness, malaise, fatigue, and weight loss. These symptoms reflect the systemic nature of the infection and the body's...
296